JMIR Publications

ABSTRACT

Background:

Mounier-Kuhn syndrome or congenital trancheobronchomegaly is a rare disease characterized by dilation of the trachea and the main bronchi. The patient's most common signs and symptoms are cough, bulky and purulent sputum, dyspnea, wheezing and recurrent respiratory infections. The presense of atrophy or absence of longitudinal elastic fibers and smooth muscle cells of the airways are findings responsible for the structural alterations found such as: tracheobronchomalacia, intercartilaginous diverticula, bulging or dilatation, and flaccidity of the airways. Specific treatment is not consensual. Inhaled bronchodilators, corticosteroids and hypertonic solution can be used. The use of non-invasive mechanical ventilation (NIMV) with continuous positive airway pressure (CPAP) is reported as a therapeutic option for tracheomalacia, but there are no prospectives studies demonstrating its efficacy.

Objective:

The Purpose is identify the presence of malacia and the possibility of finding an optimal non-invasive mechanical ventilation pressure that reduces the tracheobronchial collapse of patients with Mounier-Kuhn syndrome and to analyze the repercussion in pulmonary ventilation. In parallel, to evaluate the frequency of obstructive sleep apnoea / hypopnea syndrome.

Methods:

Clinical, transversal and open trial. Patients with diagnosed Mounier-Kuhn syndrome followed up in an outpatient clinic will be evaluate in three visits. Visit 1: registration of social demographic, clinical and functional data. Visit 2: polysomnography followed by bronchoscopy with evaluation of the presence of tracheobronchomalacia; titration of the optimal pressure capable of decreasing the collapse of the airway, concomitant with the electrical impedance tomography. Visit 3: Patients achieving reduced collapse areas will perform chest computed tomography on inspiration and forced expiration, without and with non-invasive mechanical ventilation at the titrated optimal pressure.

Results:

This study protocol is in progress. Preliminary results show that the increase in pressure is able to stabilize the airway and reverse the collapse of the trachea and bronchi.

Conclusions:

Choosing the best treatment for Mounier-Kuhn syndrome is not consensual. The use of positive pressure may be able to maintain patency of the collapsed airways, functioning as a "pneumatic stent", resulting in decreased airflow obstruction, allowing secretion mobilization, and improving pulmonary ventilation. However, study was performed to prove this hypothesis. Clinical Trial: NCT03101059